Retinoblastoma is the most frequent intraocular tumor in children under 5 years of age it is congenital, of embryonic origin, and can emerge in a multicenter pattern in one of the two retinas, derived from photoreceptors. This disease represents around 3% of the oncologic diseases that are developed by children under 15 years of age. Pathophysiology tumor is composed mainly by anaplastic cells; they are not differentiated and came from the nuclear layers of the retina. The most important clinical sign is the leukocoria that can be observed as a white reflex in the pupil. Another frequent signs is the strabismus, in particular when the macular area is involved. The differential diagnosis should be done with congenital cataract, hyperplasia of primary vitreous body and microphthalmos. In patients with transparent media, the tumor can be easily seen with an ophthalmoscope. The B-Scan Echography and the Computed Tomography is of great value. The goals of the therapy are mainly two aspect, one to cure the disease and the second to preserve as much as vision as possible. The rate of survival is relatively good. The kind of treatment will depend upon the degree of the disease inside the eye, and the verification of dissemination to some other organ. The current therapeutic focus is based upon the chemotherapy. Goal of this initial treatment is to decrease the tumor size and consolidate the mass. Then conservative focal methods are applied like photocoagulation, cryotherapy, thermotherapy and brachytherapy; it is called chemoreduction. An early diagnosis can lead to the conservation of the eye leading to a better survival rate.